Pilocytic astrocytoma and pleomorphic xanthoastrocytoma: glioneuronal tumors or variants of ganglioglioma?

OBJECTIVEBRAF V600E is a common oncogenic driver in a variety of primary brain tumors. Dual inhibitor therapy using dabrafenib (a selective oral inhibitor of several mutated forms of BRAF kinase) and trametinib (a reversible inhibitor of MEK1 and MEK2) has been used successfully for treatment of metastatic melanoma, anaplastic thyroid cancer, and other tumor types, but has been reported in only a few patients with primary brain tumors and none with pleomorphic xanthoastrocytoma. Here, the authors report on the substantial clinical response and reduction in cutaneous toxicity in a case series of BRAF V600E primary brain cancers treated with dual BRAF/MEK inhibitor therapy.METHODSThe authors treated 4 BRAF V600E patients, each with a different type of primary brain tumor (pilocytic astrocytoma, papillary craniopharyngioma, ganglioglioma, and pleomorphic xanthoastrocytoma) with the combination of dabrafenib and trametinib.RESULTSThe patients with pilocytic astrocytoma, pleomorphic xanthoastrocytoma, and papillary craniopharyngioma experienced near-complete radiographic and complete clinical responses after 8 weeks of therapy. A substantial partial response (by RANO [Response Assessment in Neuro-Oncology] criteria) was observed in the patient with ganglioglioma. The patient with craniopharyngioma developed dramatic, diffuse verrucal keratosis within 2 weeks of starting dabrafenib. This completely resolved within 2 weeks of adding trametinib.CONCLUSIONSDual BRAF/MEK inhibitor therapy represents an exciting treatment option for patients with BRAF V600E primary brain tumors. In addition to greater efficacy than single-agent dabrafenib, this combination has the potential to mitigate cutaneous toxicity, one of the most common and concerning BRAF inhibitor–related adverse events.

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Frequency and Topography of AstrocyticTumor: Experience of 567 Cases at Referral Neuroscience Hospital in Bangladesh

Journal of National Institute of Neurosciences Bangladesh ◽

10.3329/jninb.v6i2.50748 ◽

2020 ◽

Vol 6 (2) ◽

pp. 91-95

Author(s):

Md Nowfel Islam ◽

Naila Haq ◽

Md Badius Salam ◽

Monsur Ahmed ◽

Sk Muhammad Ekramullah ◽

...

Keyword(s):

Pilocytic Astrocytoma ◽

Anaplastic Astrocytoma ◽

Pleomorphic Xanthoastrocytoma ◽

Subependymal Giant Cell Astrocytoma ◽

Diffuse Astrocytoma ◽

Female Ratio ◽

Glial Tumors ◽

The Mean ◽

Male To Female ◽

Good Number

Background: Glioma is the most commonly occurring malignant brain tumor that varies by age, sex, race or ethnicity. A very few number of records on CNS tumors are available in Bangladesh. National Institute of Neurosciences and Hospital (NINS), Dhaka has a good number of CNS surgeries. Regularly both tumorous and non-tumorous ICSOL samples are examined here. Objective: The aim of the study was to see the subtypes, frequency and topography of Astrocytic tumors at NINS setting. Methodology: Data from the department of Neuropathology department of NINS since January 2013 to June 2019 were evaluated. Tissue were fixed in formalin, paraffin embedded, stained with H&E. Histomorphology and WHO 2007 CNS tumor classification were used. Result: From 3945 routine sample 567 cases were sorted out as Astrocytic tumor. Total male were 61% (346) and female 39% (221), male to female ratio was 1.6:1. The mean age was 32.64 and ranged from 1 to 80 years. Sixty six percent (66%) tumors were in supratentorial compartment, 15% infratentorial, 6.3% spinal and 9.7% in midline areas like thalamus, hypothalamus and seller region. In this study 34.6% (196) cases were Glioblastoma, followed by Anaplastic Astrocytoma 8.3%(47), Diffuse Astrocytoma 29% (165), Pilocytic Astrocytoma 26.6% (151), Pilomyxoid Astrocytoma 0.4% (2), Subependymal giant cell Astrocytoma 0.9% (5) and Pleomorphic Xanthoastrocytoma 0.1 (0.1). Topographically 66% glial tumors are supratentorial. Among the glial tumors 34.6% is Glioblastoma, 8.3% Anaplastic Astrocytoma, 29% Diffuse Astrocytoma and 26.6% Pilocytic astrocytoma. Common age group of Glioblastoma is 41-60 (52%) years, diffuse astrocytoma is 21-40 years 60.60 and Pilocytic Astrocytoma is 1-20 years (66.88). Glioblastoma, Anaplastic Astrocytoma and Diffuse astrocytoma are more common in male than female. Conclusion: There is no gender difference in case of Pilocytic Astrocytoma. Journal of National Institute of Neurosciences Bangladesh, 2020;6(2): 91-95

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Analysis of BRAF V600E mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extra-cerebellar pilocytic astrocytoma

Acta Neuropathologica ◽

10.1007/s00401-011-0802-6 ◽

2011 ◽

Vol 121 (3) ◽

pp. 397-405 ◽

Cited By ~ 643

Author(s):

Genevieve Schindler ◽

David Capper ◽

Jochen Meyer ◽

Wibke Janzarik ◽

Heymut Omran ◽

...

Keyword(s):

Nervous System ◽

Pilocytic Astrocytoma ◽

Pleomorphic Xanthoastrocytoma ◽

Braf V600e Mutation ◽

Braf V600e ◽

Nervous System Tumors

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IMG-02. USEFUL DIAGNOSIS OF PEDIATRIC CYSTIC BRAIN TUMORS USING MULTIPLE POSITRON EMISSION TOMOGRAPHY STUDIES

Neuro-Oncology ◽

10.1093/neuonc/noaa222.338 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii354-iii355

Author(s):

Keisuke Miyake ◽

Takeshi Fujimori ◽

Yasunori Toyota ◽

Daisuke Ogawa ◽

Tetsuhiro Hatakeyama ◽

...

Keyword(s):

Positron Emission Tomography ◽

Brain Tumors ◽

Pilocytic Astrocytoma ◽

Pediatric Brain Tumors ◽

Pleomorphic Xanthoastrocytoma ◽

Idh1 Mutation ◽

Emission Tomography ◽

Diffuse Astrocytoma ◽

Positron Emission ◽

Pediatric Brain

Abstract OBJECTIVE Pediatric brain tumors are primarily diagnosed using MRI or CT examination; however, determining the correct diagnosis using only morphological MRI can sometimes be challenging. Positron emission tomography (PET) uses radiotracers for metabolic and molecular imaging. We examined the accumulation of multiple PET (FDG, MET, FLT, and FMISO) studies for diagnosing pediatric cystic brain tumors. METHODS We performed PET scans for eight pediatric patients (five pilocytic astrocytoma, one pleomorphic xanthoastrocytoma, one diffuse astrocytoma with IDH1 mutation, one ganglioglioma) from April 2010 to December 2019. The resulting studies were compared by measuring the tumor-to-normal lesion (T/N) ratio of FDG, MET, and FLT and the tumor-to-blood value (T/B) ratio of FMISO between each pediatric cystic brain tumor. RESULTS All pediatric brain tumors showed tumor uptake of FDG, MET, and FLT. We could not examine FMISO PET for one diffuse astrocytoma with IDH1 mutation. The T/N ratios of FDG, MET, and FLT and the T/B ratio of FMISO were 1.07, 2.76, 4.6, and 1.12 for pilocytic astrocytoma; 0.65, 4.6, 7.67, and 1.38 for pleomorphic xanthoastrocytoma; 0.61, 2.14, and 3.82 for diffuse astrocytoma with IDH1 mutation; and 0.79, 1.78, 5, and 1.49 for ganglioglioma, respectively. The T/N ratios of MET and FLT for pleomorphic xanthoastrocytoma were high, but the Ki-67 labeling index was 1%. In the ganglioglioma, the T/N ratio of FLT was high, but the T/N ratio of MET was low. CONCLUSION Specialized multiple PET accumulation patterns for tumors are useful for discriminating each tumor.

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Astrocytic tumours

The Neurosurgeon's Handbook ◽

10.1093/med/9780198570677.003.0284 ◽

2010 ◽

pp. 381-393

Author(s):

George Samandouras

Keyword(s):

Pilocytic Astrocytoma ◽

Anaplastic Astrocytoma ◽

Pleomorphic Xanthoastrocytoma ◽

Diffuse Astrocytoma ◽

Astrocytic Tumours

Chapter 8.3 covers astrocytic tumours, including pilocytic astrocytoma (PA), diffuse astrocytoma, anaplastic astrocytoma (AA), glioblastomamultiforme (GBM), and pleomorphic xanthoastrocytoma (PXA).

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Cyclin D1 expression in ganglioglioma, pleomorphic xanthoastrocytoma and pilocytic astrocytoma

Experimental and Molecular Pathology ◽

10.1016/j.yexmp.2021.104652 ◽

2021 ◽

pp. 104652

Author(s):

Fabien Forest ◽

Pierre Dal Col ◽

David Laville ◽

Alice Court ◽

Maxime Rillardon ◽

...

Keyword(s):

Cyclin D1 ◽

Pilocytic Astrocytoma ◽

Pleomorphic Xanthoastrocytoma

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Astrocytic tumours: pilocytic astrocytoma, pleomorphic xanthoastrocytoma, and subependymal giant cell astrocytoma

10.1093/med/9780199651870.003.0002 ◽

2017 ◽

Author(s):

Brian P O’Neill ◽

Jeffrey Allen ◽

Mitchell S. Berger ◽

Rolf-Dieter Kortmann

Keyword(s):

Young Adults ◽

Giant Cell ◽

Pilocytic Astrocytoma ◽

Pleomorphic Xanthoastrocytoma ◽

World Health ◽

Subependymal Giant Cell Astrocytoma ◽

Who Grade ◽

Children And Young Adults ◽

Slow Growing ◽

Giant Cell Astrocytoma

Pilocytic astrocytoma (PA) (World Health Organization (WHO) grade I). A relatively circumscribed, slow-growing, often cystic astrocytoma occurring in children and young adults, histologically characterized by a biphasic pattern with varying proportions of compacted bipolar cells associated with Rosenthal fibres and loose-textured multipolar cells associated with microcysts and eosinophilic granular bodies. Most PAs are localized, macrocystic, and only marginally infiltrative. However some PAs, such as those arising in the optic pathways, are rarely cystic and may have an extensive infiltrative pattern but within a neuroanatomic pathway. Pleomorphic xanthoastrocytoma (PXA) (WHO grade II). An astrocytic neoplasm with a relatively favourable prognosis, typically encountered in children and young adults, with superficial location in the cerebral hemispheres and involvement of the meninges; characteristic histological features include pleomorphic and lipidized cells expressing glial fibrillary acidic protein and often surrounded by a reticulin network as well as eosinophilic granular bodies. Subependymal giant cell astrocytoma (SEGA) (WHO grade I). A benign, slow-growing tumour typically arising in the wall of the lateral ventricles and composed of large ganglioid astrocytes. It is the most common CNS neoplasm in patients with tuberous sclerosis.

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P04.04 Different stages of astrocytoma in young adult patient

Neuro-Oncology ◽

10.1093/neuonc/noz126.099 ◽

2019 ◽

Vol 21 (Supplement_3) ◽

pp. iii29-iii29

Author(s):

E Susanti ◽

R Kamarlis ◽

N Suci Rahayu

Keyword(s):

Ct Scan ◽

Pilocytic Astrocytoma ◽

Large Mass ◽

Pleomorphic Xanthoastrocytoma ◽

Low Grade ◽

Favorable Result ◽

Who Grade ◽

Head Ct ◽

Grade Ii ◽

The Brain

Abstract BACKGROUND astrocytoma is a type of cancer that involve brain parenchym or spinal cord. It arises from glial cells, called astrocytes-star shaped cells which function as supportive tissue of the brain. About 50% of primary brain tumors are astrocytomas. It’s more common in men than women and most often occur at the age of after 45. There are several types of astrocytoma and some of them grow faster than other. We found a rare case where different stages of astrocytoma occur in different areas of the brain and where the latter seemed to show up just within 8 months after the first. MATERIAL AND METHODS a 43 year old male patient admitted to neurology ward after a convulsion and hemiparesis on the left extremities. Patient was known to have suffered from astrocytoma in the frontal lobe in October 2017, and had undergone craniectomy evacuation and subsequent radiotherapy which was completed in March 2018. A few months while during recovery at home, patient complained about headache which increased in intensity over a period of 3 months before admission. The first and only convulsion occurred just 12 hours before admission, while left side weakness occurred 7 days prior. Patient underwent a series of radiologic examination after the radiotherapy and also while admitted into hospital. We found a new and large mass in the right temporoparietal lobe from the latest head ct scan. This large mass was not found from the last MRI and head ct scan which was done 8 months prior. pathology anatomy examination was done to the first and second mass found. RESULTS Pathology anatomy examination depicted different stages from the two masses, the first one was pleomorphic xanthoastrocytoma (WHO grade II) and the second one was pilocytic astrocytoma (WHO grade I) CONCLUSION pleomorphic xanthoastrocytoma (WHO grade II) and pilocytic astrocytoma (WHO grade I) both are considered low grade glioma. neverthless treatment for this devastating disease still far from favorable result or outcome

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